Get free cholesterol homeostasis and disorders in brain neurodegenerative disorder o lysosomal storage disease unesterified cholesterol o incidence 1 100000 o no approved therapies disorders of cholesterol homeostasis abstract the mevalonate mva cholesterol pathway is crucial for central nervous system cns development and . Strong evidence suggests that cholesterol also has a crucial role in the brain as various neurological and neurodegenerative disorders including alzheimers huntingtons and parkinson diseases are associated with disruptions to cholesterol homeostasis. Cholesterol synthesis in the brain brain cholesterol in adult mice is primarily supplied by de novo syntheses due to the prevention of lipoproteins uptake from the circulation by blood brain barrier bbb jeske and dietschy 1980 cholesterols metabolism in brain is separated from the rest of the body in the presence of intact blood brain barrier cellular cholesterol synthesis is a com. The mevalonate mva cholesterol pathway is crucial for central nervous system cns development and function and consequently any dysfunction of this fundamental metabolic pathway is likely to provoke pathologic changes in the brain mutations in genes directly involved in mva cholesterol metabolism cause a range of diseases many of which present neurologic and psychiatric symptoms this raises the question whether other diseases presenting similar symptoms are related albeit indirectly